Progressive stridor: extraintestinal airway manifestations in a pediatric patient with inflammatory bowel disease.

Airway manifestations of inflammatory bowel disease are rare in pediatrics. This case describes a nine-year-old female with ulcerative colitis (UC) with progressive stridor and dyspnea for two months. Severe upper airway obstruction was noted on spirometry. CatScan (CT) of the neck and chest revealed tracheal narrowing with circumferential, heterogeneous soft tissue thickening, and posterior wall nodularity. Bronchoscopy visualized the granulation tissue of the large airways and an ulcerative lesion to the right mainstem. Consultation and evaluation by gastroenterology, oncology, and rheumatology determined a diagnosis of extraintestinal manifestations of UC. Systemic steroids led to symptom resolution and improvement in lung function.

A Cross-Sectional Evaluation of Outcomes of Pediatric Thyroglossal Duct Cyst Excision.

INTRODUCTION: Thyroglossal duct cysts (TGDCs) are the most common form of congenital neck cysts. They may become infected causing dysphagia or respiratory distress. Accordingly, the treatment is always surgical removal. OBJECTIVES: The objectives of this article were to examine complications following TGDC excision by surgical specialty, demographics, and comorbid conditions. METHODS: A retrospective review of the National Surgical Quality Improvement Program database was performed. Pediatric cases from January 1, 2014 to November 1, 2015 with a current procedure terminology code of 60,280 (excision of TGDC or sinus) were included. Statistical analysis was performed to assess associations between complications and surgical specialty, demographics, and comorbidities. RESULTS: Of the 867 cases that met inclusion criteria, the median age was 4 years. There were 448 males (52.3%) and 408 females (47.7%). Thirty-six patients (4.2%) experienced at least one 30-day complication. The most predominant complications were reoperation (19 patients, 2.2%), readmission (18 patients, 2.1%), and surgical site infection (16 patients, 1.9%). There was no statistically significant difference between complications and surgical specialty. In those experiencing a complication, there was a statistically significant difference between males (86.1%) and females (13.9%). Of patients with at least one comorbidity, 36.67% had a complication, while 17.22% did not have a complication. There was also a statistically significant difference in the percentage of patients with a past medical history of asthma between those with at least one complication (16.67%) compared to those without any complications (4.76%). CONCLUSIONS: excision is a generally safe procedure across surgical specialties. There is a higher complication rate in males compared to females as well as those with a history of at least one medical comorbidity and those with asthma. The most common 30-day complications are reoperation, readmission, and surgical site infection.

Outcomes of pediatric thyroidectomy: a cross-sectional evaluation.

Introduction: Pediatric thyroidectomy is performed by a variety of surgical specialties. Thyroidectomy can result in a number of complications. Previous studies cite that the most common complications in children are pain and transient hypocalcemia. The purposes of this report are to assess the adverse events of thyroidectomies performed in the pediatric population and to assess the relationship between surgical specialties and postoperative thyroidectomy complications. Methods: We conducted a cross-sectional analysis of cases from January 1, 2014 through November 1, 2015 using the National Surgical Quality Improvement Program database for patients undergoing excision of cyst or adenoma of the thyroid, unilateral thyroid lobectomy, or total thyroidectomy. Results: Of the 344 patients who underwent thyroidectomy, 10 (2.9%) experienced at least one complication. The most common complications were readmission, surgical site infections, and wound disruption. There was a statistically significant association between complication incidence and surgical specialty (p=0.006). Pediatric otolaryngology had a statistically significantly higher number of complications than pediatric surgery (p<0.008). Conclusion: Overall, the incidence of adverse events following pediatric thyroidectomy was low.

A cross-sectional evaluation of outcomes of pediatric branchial cleft cyst excision.

OBJECTIVE: To examine complications following pediatric branchial cleft cyst excision by surgical specialty, demographics, and comorbid conditions. METHODS: A retrospective review of the National Surgical Quality Improvement Program database was performed. Pediatric cases from January 1, 2015 through May 1, 2017 with a current procedural terminology code of 42810 (excision branchial cleft cyst or vestige, confined to skin and subcutaneous tissues) or 42815 (excision branchial cleft cyst, vestige, or fistula, extending beneath subcutaneous tissues and/or into the pharynx) were included. Statistical analysis was performed to assess associations between complications and surgical specialty, demographics, and comorbidities. RESULTS: Of the 895 cases that met inclusion criteria, the median age was two years and there was an approximately equal number of males (46.8%) and females (53.2%). Forty-five patients (5.0%) experienced at least one 30-day complication, the most predominant of which was superficial surgical site infection. There was no statistically significant difference between complications and surgical specialty, complications and patient demographics, or complications and depth of excision. There was a statistically significant difference (p = 0.05) in the percentage of patients with a past medical history of developmental delay between those with at least one complication (11.1%) compared to those without any complications (4.2%). CONCLUSION AND RELEVANCE: Branchial cleft excision is a generally safe procedure across surgical specialties and patient demographics. There is an association between a history of developmental delay and 30-day postoperative complications.

Thyroglossal duct cyst surgery: A ten-year single institution experience.

OBJECTIVE: 1. Review our institution's experience with thyroglossal duct cyst (TGDC) excision. 2. Determine if TGDC recurrence rate is influenced by surgical technique, preoperative infection, or surgeon specialty. METHODS: We conducted a retrospective analysis of patients at a tertiary care children's hospital who underwent excision of neck mass with proven TGDC histopathology from 2005 to 2015. Patient demographics and presentation, preoperative workup, surgeon specialty, procedural details, lesion characteristics, and complication rates including recurrence were evaluated. RESULTS: 108 patients (51% male, 49% female), aged 6 months to 20 years (mean 6 years) met inclusion criteria. 121 procedures were performed: 96 (79.3%) for primary disease and 25 (20.7%) for recurrence. 32 patients (27.8%) were infected preoperatively, resulting in a significant increase in complications and recurrence (46.9% and 28.1%, respectively). Lack of preoperative imaging was correlated with increased recurrence (p = 0.0002). Recurrence rate after treatment for primary (11.3%) versus secondary disease (24%) was not significant (p = 0.23). Total recurrence rate differed between ENT (9.1%) and Pediatric Surgery (PS) (27.3%) (p = 0.0172). Difference was not significant for recurrence in primary disease (10% ENT, 14.8% PS, p = 0.49), but was for secondary disease (5.6% ENT, 71.4% PS, p = 0.002). Modifications of Sistrunk's procedure did not result in increased rates of recurrence (p = 0.1273). CONCLUSION: Preoperative TGDC infection and lack of imaging led to a significant increase in postoperative complications, including recurrence. Surgical specialty significantly affected recurrence rates following excision overall and in secondary disease. Recurrence was not affected by surgical technique, age, sex, cyst size, or mucin rich histology.

Pediatric Temporal Bone Fractures: A 10-Year Experience.

OBJECTIVE: The aim of the study was to compare the traditional and newer temporal bone fracture classification systems and their reliability in predicting serious outcomes of hearing loss and facial nerve (FN) injury. METHODS: We queried the medical record database for hospital visits from 2002 to 2013 related to the search term temporal. A total of 1144 records were identified, and of these, 46 records with documented temporal bone fractures were reviewed for patient age, etiology and classification of the temporal bone fracture, FN examination, and hearing status. Of these records, radiology images were available for 38 patients and 40 fractures. RESULTS: Thirty-eight patients with accessible radiologic studies, aged 10 months to 16 years, were identified as having 40 temporal bone fractures for which the otolaryngology service was consulted. Twenty fractures (50.0%) were classified as longitudinal, 5 (12.5%) as transverse, and 15 (37.5%) as mixed. Using the otic capsule sparing (OCS)/violating nomenclature, 32 (80.0%) of fractures were classified as OCS, 2 (5.0%) otic capsule violating (OCV), and 6 (15.0%) could not be classified using this system. The otic capsule was involved in 1 (5%) of the longitudinal fractures, none of the transverse fractures, and 1 (6.7%) of the mixed fractures. Sensorineural hearing loss was found in only 2 fractures (5.0%) and conductive hearing loss (CHL) in 6 fractures (15.0%). Two fractures (5.0%) had ipsilateral facial palsy but no visualized fracture through the course of the FN canal. Neither the longitudinal/transverse/mixed nor OCS/OCV classifications were predictors of sensorineural hearing loss (SNHL), CHL, or FN involvement by Fisher exact statistical analysis (for SNHL: P = 0.37 vs 0.16; for CHL: P = 0.71 vs 0.33; for FN: P = 0.62 vs 0.94, respectively). CONCLUSIONS: In this large pediatric series, neither classification system of longitudinal/transverse/mixed nor OCS/OCV was predictive of SNHL, CHL, or FN palsy. A more robust database of audiologic results would be helpful in demonstrating this relationship.

The Use of Ibuprofen in Posttonsillectomy Analgesia and Its Effect on Posttonsillectomy Hemorrhage Rate.

OBJECTIVE: To determine the effect of ibuprofen on posttonsillectomy bleeding when compared with codeine in posttonsillectomy analgesia. STUDY DESIGN: Case series with chart review. SETTING: Tertiary care children's hospital, Philadelphia, Pennsylvania. SUBJECTS AND METHODS: On July 1, 2012, our institution transitioned from acetaminophen with codeine to ibuprofen for posttonsillectomy analgesia. Pediatric patients (0-18 years old) who underwent surgery from July 1, 2010, to June 30, 2012, were placed in the codeine cohort, and those who underwent surgery from July 1, 2012, to June 30, 2014, were placed in the ibuprofen cohort. RESULTS: A total of 6014 patients underwent tonsillectomy between July 1, 2010, and June 30, 2014, and 211 patients presented for posttonsillectomy hemorrhage during the same period. The incidence of readmission for posttonsillectomy hemorrhage was 3.4% and 3.6% (P = .63; odds ratio [OR] = 1.07; 95% confidence interval [95% CI]: 0.811-1.410) for the codeine and ibuprofen groups, respectively, and the incidence of second operation for control of posttonsillectomy bleeding for the codeine and ibuprofen groups was 1.9% and 2.2% (P = .54; OR = 1.117; 95% CI: 0.781-1.600), respectively. Patients aged 11 to 18 years demonstrated a higher incidence of posttonsillectomy bleeding events overall. When age is controlled, multivariate logistic regression demonstrated no statistically significant increase in posttonsillectomy bleeding events among pediatric patients treated with ibuprofen versus patients treated with codeine (readmission: P = .617; OR = 0.932; 95% CI: 0.707-1.228; reoperation: P = .513; OR = 0.887; 95% CI: 0.618-1.272). CONCLUSION: Age is an independent risk factor for posttonsillectomy bleeding. When age is controlled, there is no statistically significant increase in the incidence of posttonsillectomy bleeding events among patients treated with ibuprofen when compared to patients treated with codeine.

Innovation in abutment-free bone-anchored hearing devices in children: Updated results and experience.

INTRODUCTION: Bone-anchored hearing devices are an accepted treatment option for hearing restoration in various types of hearing loss. Traditional devices have a percutaneous abutment for attachment of the sound processor that contributes to a high complication rate. Previously, our institution reported on the Sophono (Boulder, CO, USA) abutment-free system that produced similar audiologic results to devices with abutments. Recently, Cochlear Americas (Centennial, CO, USA) released an abutment-free bone-anchored hearing device, the BAHA Attract. In contrast to the Sophono implant, the BAHA Attract utilizes an osseointegrated implant. OBJECTIVES: This study aims to demonstrate patient benefit abutment-free devices, compare the results of the two abutment-free devices, and examine complication rates. METHODS: A retrospective chart review was conducted for the first eleven Sophono implanted patients and for the first six patients implanted with the BAHA Attract at our institution. Subsequently, we analyzed patient demographics, audiometric data, clinical course and outcomes. RESULTS: Average improvement for the BAHA Attract in pure-tone average (PTA) and speech reception threshold (SRT) was 41dB hearing level (dBHL) and 56dBHL, respectively. Considering all frequencies, the BAHA Attract mean improvement was 39dBHL (range 32-45dBHL). The Sophono average improvement in PTA and SRT was 38dBHL and 39dBHL, respectively. The mean improvement with Sophono for all frequencies was 34dBHL (range 24-43dBHL). CONCLUSION: Significant improvements in both pure-tone averages and speech reception threshold for both devices were achieved. In direct comparison of the two separate devices using the chi-square test, the PTA and SRT data between the two devices do not show a statistically significant difference (p-value 0.68 and 0.56, respectively). The complication rate for these abutment-free devices is lower than that of those featuring the transcutaneous abutment, although more studies are needed to further assess this potential advantage.

Epithelioid hemangioma of the internal carotid artery: a case report supporting the reactive pathogenesis hypothesis of this vascular tumor.

The pathogenesis of epithelioid hemangioma is intriguing and has remained a controversial topic in the medical literature. Whether its etiology follows a neoplastic or reactive process is unclear, but a history of traumatic insult to the involved region is common. We report a case of epithelioid hemangioma of the internal carotid artery in a child who had undergone cannulization of the internal jugular vein as an infant to receive extracorporeal membrane oxygenation.

Abutment-free bone-anchored hearing devices in children: initial results and experience.

OBJECTIVES: Bone-anchored implantable hearing devices are widely accepted as a surgical option for certain types of hearing loss in both adults and children. Most commercially available devices involve a percutaneous abutment to which a sound processor attaches. The rate of complications with such bone conduction systems is greater than 20%. Most complications arise from the abutment. Recently, the Sophono (Boulder, CO) Alpha 1, an abutment-free system, has been introduced. STUDY DESIGN AND METHODS: We conducted a retrospective chart review of the first five patients who underwent implantation with the Sophono abutment-free bone conduction hearing system with the Alpha 1 processor at our institution and report here on these patients' pre- and postoperative audiometric data and clinical courses. RESULTS: Average improvement in pure-tone average was 32dB hearing loss and average improvement in speech response threshold was 28dB hearing loss. All patients were responding in the normal to mild hearing loss range in the operated ear after device activation. Average improvement across individual frequencies was between 17 and 37dB (SD 5.5-11dB). CONCLUSION: Our audiometric results to date are promising and have been consistent with published data on other bone-anchored hearing devices.

Hearing loss in children with mitochondrial disorders.

OBJECTIVE: At least 1-5 children per 1000 suffer from congenital hearing loss, and 50% of these cases can be attributed to genetic causes. It has been estimated that 1% of pre-lingual hearing loss is due to mutations in mitochondrial DNA. Previous literature reports audiometric data for few patients, usually less than 20 per study. The goal of this study was to characterize the hearing loss associated with mitochondrial mutations and determine whether previously characterized patterns of hearing loss in these patients (progressive, sensorineural, high frequency losses) are found in our population as well. METHODS: An IRB-approved retrospective chart review of the electronic medical records in the Nemours/Alfred I. dupont Hospital for Children system from January 2004 to October 2009 (a five-year period) was undertaken using ICD-9 codes 277.87 (mitochondrial disorder) and 359.89BA (mitochondrial myopathy). These 149 records were then evaluated for audiologic data, resulting in 26 charts with both a mitochondrial disorder and hearing evaluation. RESULTS: Of 26 patients with known mitochondrial disorders and audiometric documentation, 15 (58%) had hearing loss, and 11 patients had normal hearing (42%). Ten patients had sensorineural hearing loss (38%), two patients had conductive hearing loss (7.7%), one patient had a mixed hearing loss (3.8%), and two patients had an as yet undefined hearing loss (ABR had not yet been performed at the time of this study) (7.7%). CONCLUSION: In comparison with previous studies, generally including less than 20 patients, this is one of the largest collections of audiometric data on children with mitochondrial disorders. Unlike prior studies describing a progressive, sensorineural loss across all frequencies or mainly affecting high frequencies, the hearing loss in our patients was more variable including low frequency losses, mid-frequency losses, and conductive losses and was often not progressive or even improved. Our overall 38% rate of sensorineural hearing loss correlates well with previous case series; this study clearly justifies the use of routine audiometric screening in children with mitochondrial disorders, including use of ABR and OAEs as ASND can be seen in this population, as well as repeat testing over time to evaluate for progression.

Adult Burkitt lymphoma originating in the sphenoid sinus: case report and review of the literature.

Burkitt lymphoma is a high-grade B-cell non-Hodgkin lymphoma. The endemic form of this malignancy occurs primarily in children aged 5 to 7 years, and it presents with jaw and facial bone involvement. The sporadic form affects older children (mean age: 12.2 yr) and often manifests as an abdominal mass; it rarely involves the head and neck. The presence of any type of lymphoma in the paranasal sinuses is rare. We report a case of Burkitt lymphoma that originated in the sphenoid sinus in a 66-year-old white woman. The patient presented with hypoesthesia in the left V1 and V2 distributions and frequent left-sided headaches. Imaging revealed that the destructive lesion had spread into the cavernous sinus and infratemporal fossa. Repeat imaging showed progression of the lesion in just 1 month. An endoscopic sphenoidotomy was performed to obtain a tissue specimen, and a diagnosis of sporadic Burkitt lymphoma was established on the basis of its clinical, morphologic, and immunohistochemical characteristics. The patient underwent chemotherapy and radiation. Short-term follow-up imaging showed that the lesion had disappeared, and the patient remained disease-free at 3 years of follow-up. This case is one of the few reported cases of true adult Burkitt lymphoma originating in the sphenoid sinus. We discuss the rapid progression of the disease and the considerable amount of invasion that can occur with minimal symptoms.

Effects of an LL-37-derived antimicrobial peptide in an animal model of biofilm Pseudomonas sinusitis.

BACKGROUND: LL-37, an innate immunity protein expressed within sinonasal mucosa, has in vitro antibacterial and antifungal properties as well as efficacy against preformed Pseudomonas aeruginosa biofilms. We hypothesize that a 24 amino acid peptide derivative of LL-37 will show efficacy against biofilm-forming P. aeruginosa in an established animal model of sinusitis. METHODS: Five groups of six New Zealand rabbits were each infected with P. aeruginosa (PAO-1) and fitted with irrigating catheters 7 days later. Each group was instilled with either one of three different concentrations of peptide, a positive control of topical tobramycin, or the carrier solution without the peptide once a day for 10 days. Nasal diluent was collected throughout the irrigation period to assess for persistence or resolution of infection by determining colony-forming units (CFU). At study end, sinus mucosa was harvested for histological assessment of inflammation and SEM evaluation for ciliary integrity and presence of biofilms. RESULTS: Topical tobramycin at 400x minimum inhibitory concentration and 2.5 mg/mL of peptide were effective in significantly lowering CFUs after 10 days of irrigation. Histological evaluation showed increased signs of inflammation in a dose-dependent manner within mucosa and bone of the groups receiving the peptide. SEM analysis showed ciliary loss in a dose-dependent manner. Biofilms were present in all groups except for the highest concentration of peptide and tobramycin. CONCLUSION: High concentrations of LL-37-derived peptide showed in vivo ability to eradicate Pseudomonas biofilms and decrease bacterial counts. However, increasing concentrations of peptide showed proinflammatory and ciliotoxic effects on sinus mucosa.

Intraoperative IGS/CT updates for complex endoscopic frontal sinus surgery.

INTRODUCTION: Computer-aided surgery (CAS) systems integrate endoscopic visualization with real-time localization based on preoperative imaging. One short-coming of the current systems is the lack of real-time, radiographic assessment of surgical changes. We describe the potential applications of xCAT, a new intraoperative mobile volume CT scanner and its utility in providing surgical navigation updates in the operating room. METHODS: A case report is presented describing how intraoperative CT updates were used in a complicated, revision endoscopic frontal sinus surgery. RESULTS: A 53-year-old male with Samter's triad and a history of multiple previous endoscopic sinus surgeries presented with recurrent, symptomatic nasal polyposis and right-sided frontal headaches. Preoperative CT scans showed a large type III frontal recess cell obstructing the frontal sinus. He underwent revision endoscopic surgery with the use of CAS and the xCAT intraoperative CT scanner. Use of the intraoperative CT scanner to update preoperative images was extremely helpful in the endoscopic dissection of the frontal recess cell and in assessing completeness of dissection along the anterior skull base. CONCLUSION: The xCAT intraoperative mobile volume CT scanner shows promise in providing real-time updates to surgical navigation. This may prove to be a valuable tool in endoscopic sinus surgery, especially in complex revision and frontal sinus cases.

Osteosarcoma of the skull base: case report and review of literature.

Osteosarcoma is the most common primary malignancy of bone in children and adolescents. Osteosarcomas are an aggressive neoplasm composed of spindle cells producing osteoid. They primarily affect the long bones, particularly after radiation or chemotherapy for other neoplasms; however, 6-7% present in the head and neck. Primary head and neck osteosarcomas in children are rare. There are few case reports and limited-sized case series in the literature. A case report presentation of a skull base osteosarcoma in a teenage female. A 14-year-old African American female presented with dysphagia, voice changes, and neck pain. On examination, she had right-sided palsies in cranial nerves X, XI, and XII. Imaging revealed partial enhancement of the clivus without bony erosion and expansion of the hypoglossal canal. There were also findings consistent with chronic denervation of her right tongue and pharynx. During the evaluation process, she developed diplopia from a right cranial nerve VI palsy. Repeat imaging revealed progression of the skull base lesion with extension into the right sphenoid sinus. An endoscopic sphenoidotomy was performed to obtain tissue. The diagnosis of high-grade osteosarcoma was made by histologic morphology and immunohistochemistry. The child was treated primarily with chemotherapy. Other adjunctive therapies are being considered. Osteosarcoma of the skull base is a rare entity. We describe a case of a high-grade clival osteosarcoma presenting primarily with lower cranial nerve palsies and pain. The rapid progression, treatment options, and prognosis are discussed.

Microdebrider decompression of schwannoma: a novel method of excising a neck mass.

OBJECTIVE: This case report describes the excision of a large neck neurofibroma causing compression of the esophagus and airway in a young patient with neurofibromatosis type 2 (NF2). At the conclusion of this article, readers should be able to describe a novel method of excising large encapsulated neck masses using microdebridement for decompression. The safety and efficacy of this method is discussed. STUDY DESIGN: The subject of this report was a 26-year-old woman with NF2. She presented with an enlarging right neck mass extending from the mandible to the clavicle that was compressing both her airway and esophagus. Given her auditory brainstem implant, unipolar cautery was contraindicated. Therefore, it was planned to decompress the patient's neck mass using a microdebrider before attempting to fully dissect out the mass. METHODS: The neck mass was exposed and then entered. Using a Xomed XPS microdebrider (Medtronic Inc., Minneapolis, MN), the mass was debrided and debulked in all directions taking care not to violate the capsule. After this, the entire capsule was dissected out using only bipolar cautery and suture ligatures for hemostasis. RESULTS: Microdebrider decompression of the neck neurofibroma allowed for preservation of the capsule without injuring vital structures in the neck. Postoperatively, the patient's swallowing and laryngeal function improved markedly. CONCLUSION: Microdebrider debulking before dissection of the patient's large neck mass safely relieved compression of the airway and esophagus. This method may be applied to other large benign masses in the neck as well.

Clinical predictors for hearing loss in children with bacterial meningitis.

OBJECTIVES: To identify clinical risk factors that predict a higher incidence of hearing loss in children with bacterial meningitis, to determine the overall incidence of hearing loss in a large group of children proven by culture findings to have bacterial meningitis, and to compare clinical characteristics among patients with Streptococcus pneumoniae meningitis and Neisseria meningitidis meningitis. DESIGN: Retrospective review SETTING: Tertiary pediatric hospital. PATIENTS: A total of 171 children identified with bacterial meningitis who met inclusion criteria over a consecutive 10-year period. MAIN OUTCOME MEASURE: Presence of sensorineural hearing loss. RESULTS: Of 134 patients who underwent audiologic testing during their initial hospitalization, 41 (30.6%) were found to have at least a unilateral mild sensorineural hearing loss. The incidence of hearing loss was greater in patients with S pneumoniae meningitis than in patients with N meningitidis meningitis (35.9% and 23.9%, respectively). Length of hospitalization, development of seizures, elevated cerebrospinal fluid protein, and decreased cerebrospinal fluid glucose were significant predictors for hearing loss in children with bacterial meningitis. These factors were not found to be as strong a predictor for hearing loss in patients with N meningitidis meningitis. Stability of hearing was demonstrated with limited follow-up audiometry. CONCLUSIONS: Sensorineural hearing loss is a common sequela in children with bacterial meningitis. Identification of hearing loss in children with bacterial meningitis and early rehabilitation will lessen the long-term educational and social difficulties these children may experience.